A 64-year-old man presented to pain in the left upper quadrant and weight loss. Findings on magnetic resonance imaging (MRI) of the abdomen included the filling of mass in whole pancreatic ducts with marked dilatation (Figure 1A,B, within yellow dots). Total pancreatectomy was performed and whole pancreas replaced by intraductal mass like lesion (Figure 1C). Mural nodules filled with the whole pancreas. Pathologic result revealed intraductal tubulopapillary neoplasm (ITPN) with invasive carcinoma. At a follow-up 4 years after surgery, he died with polycystic kidney infection without evidence of ITPN recurrence.
ITPN is a rare pancreatic tumor belonging to a newly recognized entity that is updated in the 2010 WHO classification. It is definite difference between intraductal papillary mucinous neoplasm (IPMN). There were few reports of IPMN with polycystic kidney and liver disease but it is first report ITPN with polycystic kidney and liver disease on the literature.
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